Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
J Cutan Pathol. 2020 Nov;47(11):1050-1053. doi: 10.1111/cup.13791. Epub 2020 Sep 10.
Acral localized acquired cutis laxa (ALACL) is a rare variant of acquired cutis laxa, and the clinical appearance is characterized by loose, redundant and wrinkled skin of the distal extremities. By definition, histopathology of affected tissue reveals sparse or fragmented elastic fibers. However, this can be difficult to assess on routine staining, and sometimes requires electron microscopy. The condition has been associated with plasma cell dyscrasias or recurrent inflammatory states. We present a case of a 65-year-old man who presented with enlarged and doughy finger pads. Skin biopsy showed diffuse dermal amyloid deposition displacing dermal stroma and reduction of elastic fibers, although these changes were subtle on routine hematoxylin and eosin staining. Mass spectrometry of laser capture microdissected tissue showed AL kappa-type amyloid and further workup revealed a diagnosis of primary systemic AL-kappa amyloidosis requiring bone marrow transplantation. This case represents an unusual presentation of acquired cutis laxa and highlights the need for a high index of suspicion when reviewing histopathology of this entity. In addition, the case highlights the importance of investigation into possible systemic associations, such as plasma cell dyscrasias.
肢端局限性获得性皮肤松弛症（ALACL）是获得性皮肤松弛症的一种罕见变异，其临床特征为远端肢体皮肤松弛、多余和起皱。根据定义，受累组织的组织病理学显示稀疏或碎片化的弹性纤维。然而，这在常规染色时很难评估，有时需要电子显微镜检查。该病症与浆细胞异常或复发性炎症状态有关。我们报告了一例 65 岁男性，其表现为指垫增大和面团状。皮肤活检显示弥漫性真皮淀粉样物质沉积，取代真皮基质并减少弹性纤维，尽管这些变化在常规苏木精和伊红染色时很细微。激光捕获显微解剖组织的质谱分析显示 AL kappa 型淀粉样蛋白，进一步检查显示原发性系统性 AL-kappa 淀粉样变性的诊断需要骨髓移植。该病例代表了获得性皮肤松弛症的一种不常见表现，并强调了在审查该实体的组织病理学时需要高度怀疑。此外，该病例强调了调查可能的全身关联（如浆细胞异常）的重要性。
